Mutation Story

Part 1

People always used to make fun of me and call me names just because I was Different. I started out life thinking I was just a normal muscle cell, but when my host Bob turned two there was something different going on with his body. Everyone blamed this on me. At first I didn’t know what was going on but I eventually learned that Bob was aging very quickly. He had progeria and it was all because of my DNA.


When my cell was born the DNA sequence was different and I was left with a deletion of a base called thymine, which Made me be born with the gene progerin. Progerin is an abnormal lamin A protein that causes progeria. Me not knowing what I was doing wrong, just thinking I was just doing normal muscle cell functions, but I was producing this protein that causes the nucleus to be unstable. This cellular instability leads to the process of premature aging and the disease progeria. And of course I can’t stop this.


Like I said when I was younger I was the odd man out but know there is many duplicates of me, because mitosis has occurred many times and my genes have been duplicated. At first me and my cell friends thought this disease was good because Bob was smarter than everyone else. But we learned this is a negative mutation. I also heard I-4 million people are diagnosed with progeria and unfortunately Bob will die at around 14. Bob’s death will probably occur from a heart attack or stroke because of this fast aging. His joints will be stiff, he will be bald, and he will just look like an old man all because of me. Of course I was depressed and mad about this at first, but their is hope for Bob and other children with progeria.


The PRF stands for Progeria Research Foundation and its main goal is to help children with progeria live a long and happy life. Bob has had to go to many meetings with PRF and of course I have gone along with him. At these meetings in Boston at PRF’s headquarters Bob not only gets to help the foundation by letting them give him test treatments like lonafarnib that is a type of farnesyltransferase inhibitor, but he gets to meet other kids with progeria. This makes Bob and me feel a lot better about ourselves. The treatment that Bob has been tested on actually was proven to help with making him have a steady weight, bone structure, hearing and the flexibility of blood vessels. This doesn’t mean that Bob and I will live till we’re old but we want to help other kids with progeria for the future. Me and Bob only have an estimated 2 years left in our lives because there is no real cure for progeria, but we are so thankful for the PRF studies and hope that one day kids with progeria will be benefited from our testings so they can live till they’re old.


Picture Resources

Part 2

Guiding Questions:

  • What is progeria?
  • What causes progeria?
  • How common is progeria?
  • Who researches around progeria?
  • Is there a cure for progeria?
  • Why is progeria a negative mutation?
  • Does progeria spread?
  • What are the features of progeria?
  • What does progeria have to do with aging?
  • What is PRF doing to help kids with progeria?

The tools I used to find information:

  • Riverside digital library
  • PRF site
  • My father because he knows someone who has a mutation
  • My science teacher
  • Website comments

The process I used to investigate my topic:

  • Found good resources
  • made sure the information was true
  • Answered my questions from the information
  • Put the information into my own words
  • made the story interesting

How I verified and cited the information:

  • Made sure I found it in riversides digital library
  • Looked through the cites info
  • Looked through comments on the cite
  • Looked through ratings on the cite
  • Made sure other cites had the same info

How I found the process of creating this project:

  • The process throughout me making this project was actually pretty interesting. I got to teach myself about this mutation by answering these questions I made for myself at the beginning of this project. This made it very fun because I figured out my problems and this made me fell accomplished when I finished the project. Using the different digital tools was also very cool to because I got to try out new things. I think improvements could have been made to my projects by finding more resources and further researching my project. Also I could have created more questions for myself to answer. In conclusion I believe this project was very fun and I would like to do something like this again.

BC Power Story

         In today’s world electricity is a thing that everybody relies on and can’t live without but how is electricity really created and brought to your house. In Port Coquitlam most power is generated from either the hydroelectric generation in Buntzen lake or thermal generation in the Burrard inlet. The way these work are different and there’s pros and cons to the way both power generators work and that is what I’m going to tell you about today.

         Hydroelectric generation is using water as a fuel. A dam raises the water level of the river to create falling water which forms a reservoir. The dam can control the flow of water which is how much water that can go through the turbines. These turbines are near the bottom of the dam where water can flow through. Attached to the turbines are blades causing the turbine to spine because of the pressure of the water. A water turbine is much like a windmill using water to spin the blades instead of wind. The turbine converts the kinetic energy of the water into mechanical energy. A generator is connected to the turbine by shafts so that when turbine spines the generator also spins, this converts the mechanical energy from the turbine into electrical energy. Of course there are many pros and cons to this process and some pros are that it is renewable, which means we can’t use the water up. Another pro is that this is a very reliable way of creating energy and there is no pollution. Also this process is very safe because the only fuel is water. Some cons are environmental consequences such as water flow, damning water, construction of roads and power lines. The biggest con I believe is the affect on fish because it changes there habitat such as shelter, food, water velocity and lifecycle. Hydroelectric generation is also very expensive, droughts could stop water flow and it is hard to find good spots to build these damns.

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Thermal Generation is a more dirty way of creating electricity because you have to burn natural gas as a fuel. This natural gas is burning water in boiling tank to create steam. The superheated steam drives a turbine in a circular shaft which creates kinetic energy. This drives the generator to turn kinetic energy into electrical energy. The pros of creating energy through thermal generation are that fossil fuels are the easiest form of energy that can be found in the earths crust. These fuels can easily be transported through pipelines or in semi trucks. Also this type of electricity generation gives lots of employment. The biggest con to thermal generation is of course the pollution which creates greenhouse gases and if we use up all the fossil fuels there won’t be any left for the next generation to come. Also these work places are usually pretty unsafe and the waste from the plants are hazardous and can go into our wild.

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Now we know how electricity is generated but how does it get to our houses? Well after electricity goes through a generator at the power plant. In order for the transmission lines to carry the electricity over a long distance the voltage must be bumped up by a Step-up transformer which has two coils inside and a iron core.The secondary coil has more turns of wire than the primary coil. Which makes the output twice as much as the input because the secondary coil has twice as many turns of wire than the primary coil. Next the electricity is passed through high voltage transmission lines which usually contains one or more conductor. These high voltage transmission lines are supported by tall metal towers carrying high voltage electricity over long distances. After that the electricity goes to a terminal station to control the power flow on grid transmission lines and reduce the grid voltage to sub transmission voltage levels using a step down transformer which also has two coils and an iron core but the secondary coil has less as many turns as the primary coil so the output is smaller than the input. Then this electricity is passed on to sub transmission lines that run at lower voltages to large industrial costumers or large distribution stations for heavy equipment. These lines are usually held up by an “H” frame made out of wood and also use conductors to transfer the electricity. After that the electricity is brought to a distribution station where electricity is lowered again by more step down transformers and is in a system of meters, control and protective devices. Then this electricity can finally be brought to our houses at a safe electricity level at 120,240V by low voltage power lines. In conclusion that is how power is generated in our area and how it is brought to our houses. Now we finally now about this great thing we cannot live without!

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By Anthony Van Hulsen